Clinical Bedside Tools to Assess Systemic Sclerosis Vasculopathy: Can digital thermal monitoring and sublingual microscopy identify patients with digital ulcers?

Objective Sublingual microscopy assesses systemic sclerosis (SSc) vasculopathy. Digital thermal monitoring (DTM) may identify patients at risk for digital ulcer (DU). The purpose of this analysis was to assess sublingual microscopy and DTM in SSc patients with and with no previous DU in order to determine the utility of these clinical tools. Methods SSc registry patients with clinical data had both DTM and sublingual microscopy on the same day were included in this cross-sectional analysis. DTM quantifies vascular reactivity index (VRI). Sublingual microscopy measures longitudinal red blood cell fraction (RBC fract) and perfused boundary region (PBR). We evaluated the pairwise association between VRI, RBCfract and PBR in a monotonic relationship using Spearman’s rank correlation in the DU subset. Correlation coefficients (rs) and their 95% confidence intervals (CIs) were reported. Results Ninety patients were included; 29 had digital pits and/or active DU and 61 never had a DU. The only significant clinical feature associated with DU was modified Rodnan skin score (p=0.003) with DU being higher. The VRI was lower in patients with DU (p=0.01). The higher RBCfract the lower PBR (rs =- 0.71, 95% CI: -0.86, -0.47, p<0.001). VRI was not associated with RBCfract or PBR (p=0.24 or 0.55, respectively) in the DU patients. Conclusion DTM is a useful tool for assessing SSc-DU. While sublingual microscopy measurements did not significantly correlate to VRI in SSc-DU patients, a longitudinal study may be more helpful in capturing vasculopathy activity prior to possibly irreversible damage.

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Raynaud Phenomenon in Systemic Sclerosis: Does Digital Thermal Monitoring Correlate to Specific Nailfold Videocapillaroscopy Abnormalities?

The Journal of Rheumatology ◽

10.3899/jrheum.191371 ◽

2020 ◽

pp. jrheum.191371

Author(s):

Julie Thomas ◽

Mislav Radic ◽

Jordan R. Tucker ◽

Rebecca Overbury ◽

Tracy M. Frech

Keyword(s):

Systemic Sclerosis ◽

Vascular Function ◽

Vascular Reactivity ◽

Clinical Care ◽

Density Measurement ◽

Nonparametric Tests ◽

Reactivity Index ◽

Thermal Monitoring ◽

Nailfold Videocapillaroscopy ◽

Raynaud Phenomenon

Objective Early diagnosis of systemic sclerosis (SSc) is imperative, and Raynaud phenomenon (RP) is an important component of progressive vasculopathy. Nailfold videocapillaroscopy (NVC) is a well-established tool that can quantify structural vascular abnormalities. Digital thermal monitoring (DTM) assesses microvascular functional dysfunction related to thermoregulation. In this study, we investigated the correlation of NVC patterns and DTM variables in patients with SSc. Methods Patients with SSc according to the 2013 American College of Rheumatology/European League Against Rheumatism criteria who consented and enrolled in the clinical care registry had NVC and DTM performed. For NVC, the number of capillaries (density), measurement of apical diameter (dimension), presence or absence of hemorrhages, and number of abnormal shapes were assessed to categorize 3 different qualitative patterns: early, active, and late. For DTM, Doppler ultrasound hyperemic, low frequency, blood velocity of radial artery, and fingertip vascular function were assessed, and a vascular reactivity index (VRI) measurement was automated. Statistical evaluation was performed by nonparametric tests to assess the correlation of NVC and VRI. Results Thirty-one SSc subjects with interpretable NVC and DTM performed on the same day were included in the study. VRI was progressively higher in SSc patients with early, active, and late NVC patterns of microangiopathy (P < 0.0001). There was a significant negative correlation between VRI and microhemorrhages scores (r = –0.363, P = 0.044). Conclusion Our study suggests that more advanced vasculopathy correlates to reduced microvascular function as detected by DTM and more advanced structural abnormalities detected by NVC. NVC and DTM may provide different aspects of vasculopathy quantification and complement each other as investigative tools.

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Serum Adipocyte Fatty Acid-Binding Protein Level is Negatively Associated with Vascular Reactivity Index Measured by Digital Thermal Monitoring in Kidney Transplant Patients

Metabolites ◽

10.3390/metabo9080159 ◽

2019 ◽

Vol 9 (8) ◽

pp. 159 ◽

Cited By ~ 2

Author(s):

Tai-Li Chen ◽

Ming-Che Lee ◽

Ching-Chung Ho ◽

Bang-Gee Hsu ◽

Jen-Pi Tsai

Keyword(s):

Fatty Acid ◽

Endothelial Function ◽

Kidney Transplant ◽

Vascular Reactivity ◽

Fatty Acid Binding Protein ◽

Reactivity Index ◽

Thermal Monitoring ◽

Fatty Acid Binding ◽

Negatively Associated ◽

Acid Binding Protein

Adipocyte fatty acid-binding protein (A-FABP) is abundantly found in mature adipocytes and is involved in cardiovascular disease. Our aim is to investigate the association between serum A-FABP levels and endothelial function among kidney transplant (KT) patients. Fasting blood samples were obtained from 80 KT patients. Serum A-FABP levels were measured using a commercially available enzyme immunoassay kit. Endothelial function and vascular reactivity index (VRI) were measured using digital thermal monitoring test. In this study, VRI < 1.0, VRI 1.0–1.9, and VRI ≥ 2.0 were defined as poor, intermediate, and good vascular reactivity, respectively. There were 12 (15.0%), 30 (37.5%), and 38 (47.5%) KT patients categorized as having poor, intermediate, and good vascular reactivity, respectively. Increased serum levels of alkaline phosphatase (p = 0.012), γ-glutamyltranspeptidase (GGT; p = 0.032), and A-FABP (p < 0.001) were associated with decreased vascular reactivity. Multivariable forward stepwise linear regression analysis revealed that age (β = −0.283, adjusted R2 change = 0.072; p = 0.003) and serum log-A-FABP level (β = −0.514, adjusted R2 change = 0.268; p < 0.001) were significantly associated with VRI values in KT patients. We concluded that serum fasting A-FABP level is negatively associated with VRI values and plays a role in endothelial dysfunction of KT patients.

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Automated Measurement of Microvascular Function Reveals Dysfunction in Systemic Sclerosis: A Cross-sectional Study

The Journal of Rheumatology ◽

10.3899/jrheum.170120 ◽

2017 ◽

Vol 44 (11) ◽

pp. 1603-1611 ◽

Cited By ~ 11

Author(s):

Daniel R. Machin ◽

Phillip E. Gates ◽

Hans Vink ◽

Tracy M. Frech ◽

Anthony J. Donato

Keyword(s):

Systemic Sclerosis ◽

Barrier Properties ◽

Boundary Region ◽

Cross Sectional Study ◽

Microvascular Density ◽

Endothelial Glycocalyx ◽

Inverse Association ◽

Healthy Controls ◽

Cross Sectional ◽

Sublingual Microcirculation

Objective.The aim of our study was to determine whether an automated capture and analysis system could detect differences in structure and function of sublingual microvessels in patients with systemic sclerosis (SSc) compared to healthy controls.Methods.Intravital microscopy of the sublingual microcirculation was automatically captured and analyzed in 40 patients with SSc and 10 age-matched healthy controls.Results.Total and perfused microvascular density were lower in patients with SSc compared with controls (total microvascular density: 2471 ± 134 µm/mm2 vs 3067 ± 197 µm/mm2, p = 0.020; perfused microvascular density: 1708 ± 92 µm/mm2 vs 2192 ± 144 µm/mm2, p = 0.009). However, the relative percentage of perfused to total microvascular density was similar between SSc and controls (72 ± 2% vs 71 ± 2%, respectively, p = 0.429). Mean red blood cell (RBC) fraction, which indicates the longitudinal tube hematocrit of microvessel segments, was lower in patients with SSc compared with controls (69 ± 1% vs 77 ± 1%, respectively, p < 0.001). Perfused boundary region (PBR), a marker of endothelial glycocalyx barrier properties, was higher in patients with SSc compared with controls (2.1 ± 0.0 µm vs 1.9 ± 0.0 µm, respectively, p = 0.012), suggestive of a dysfunctional glycocalyx. There was an inverse association of PBR with perfused microvascular density (r = −0.40, p = 0.004) and RBC fraction (r = −0.80, p < 0.001).Conclusion.Our results indicate that automated capture and analysis of sublingual microvessel segments produces detailed, objective microvascular structural and functional data that have allowed us to distinguish patients with SSc from controls. These data suggest that microvascular structural and functional abnormalities present in patients with SSc could be at least partly due to a dysfunctional glycocalyx.

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New Indices of Endothelial Function Measured by Digital Thermal Monitoring of Vascular Reactivity: Data from 6084 Patients Registry

International Journal of Vascular Medicine ◽

10.1155/2016/1348028 ◽

2016 ◽

Vol 2016 ◽

pp. 1-8 ◽

Cited By ~ 7

Author(s):

Morteza Naghavi ◽

Albert A. Yen ◽

Alex W. H. Lin ◽

Hirofumi Tanaka ◽

Stanley Kleis

Keyword(s):

Blood Pressure ◽

Endothelial Function ◽

Diastolic Blood Pressure ◽

Vascular Reactivity ◽

Cardiovascular Health ◽

Patient Characteristics ◽

Reactivity Index ◽

Thermal Monitoring ◽

Reactivity Measurement ◽

Large Patient

Background. Endothelial function is viewed as a barometer of cardiovascular health and plays a central role in vascular reactivity. Several studies showed digital thermal monitoring (DTM) as a simple noninvasive method to measure vascular reactivity that is correlated with atherosclerosis risk factors and coronary artery disease. Objectives. To further evaluate the relations between patient characteristics and DTM indices in a large patient registry. Methods. DTM measures were correlated with age, sex, heart rate, and systolic and diastolic blood pressure in 6084 patients from 18 clinics. Results. DTM vascular reactivity index (VRI) was normally distributed and inversely correlated with age (r=-0.21, p<0.0001). Thirteen percent of VRI tests were categorized as poor vascular reactivity (VRI < 1.0), 70 percent as intermediate (1.0 ≤ VRI < 2.0), and 17 percent as good (VRI ≥ 2.0). Poor VRI (<1.0) was noted in 6% of <50 y, 10% of 50–70 y, and 18% of ≥70 y. In multiple linear regression analyses, age, sex, and diastolic blood pressure were significant but weak predictors of VRI. Conclusions. As the largest database of finger-based vascular reactivity measurement, this report adds to prior findings that VRI is a meaningful physiological marker and reflects a high level of residual risk found in patients currently under care.

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SAT0327 SEXUAL DYSFUNCTION IN FEMALE SCLERODERMA PATIENTS AND ITS CORRELATION WITH VASCULAR INVOLVEMENT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4084 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1109.1-1109

Author(s):

Z. Khodamoradi ◽

M. Nazarinia ◽

E. Esmaeilzadeh

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Sclerosis ◽

Sexual Dysfunction ◽

Vascular Complications ◽

Vascular Involvement ◽

P Value ◽

Case Group ◽

Digital Ulcers ◽

Cross Sectional ◽

Female Patients

Background:Systemic Sclerosis (Scleroderma, SSc) is an autoimmune disorder characterized by multi-organ dysfunction, which ultimately leads to multiple clinical and psychological complications. Among various complications of scleroderma, sexual dysfunction can be named as a major issue in both male and female patients, which has great impact on quality of life of the patients.Objectives:Investigating the sexual dysfunction in scleroderma patients and its relation to their vascular involvements.Methods:A case control study was done on 80 married female scleroderma patients with age between 20-60 years old. Eighty normal individuals adjusted for age, place of living and socioeconomic status were also recruited. Sexual performance in both groups was assessed using FSFI standardized questionnaire, which evaluated it in 6 domains of desire, arousal, lubrication, orgasm, satisfaction, and pain. Micro and macro-vascular involvements of the patients were also determined using Raynaud Condition Score, Echocardiography, physical exam for assessing their digital ulcers and reviewing their medical records for presence of past or present history of renal crisis and thromboembolic events.Results:The total score of FSFI in the case group was significantly lower compared to control one (16.68 ± 6.35, 19.69 ± 6.01, P-value <0.001). The score was significantly lower in all domains of sexual dysfunction except for pain and lubrication. Moreover, the mean score of FSFI was also found to be significantly lower in limited form of the disease compared to diffuse one (14.6 ± 6.9, 18.1 ± 5.5, P-value 0.01). No significant association was found between vascular complications and sexual impairment of the scleroderma patients.Conclusion:This study can be named as the first survey investigating the sexual dysfunction in Iranian female scleroderma patients and assessing its relation with vascular complication of the disease. Thus, it can be a guide for future studies on sexual dysfunction especially in societies with cultural limitations in discussing this issue.References:[1]Bruni C, Raja J, Denton CP, Matucci-Cerinic M. The clinical relevance of sexual dysfunction in systemic sclerosis.Autoimmun Rev2015; 14(12):1111-5. doi: 10.1016/j.autrev.2015.07.016.[2]Puchner R, Sautner J, Gruber J, Bragagna E, Trenkler A, Lang G. et al. High Burden of Sexual Dysfunction in Female Patients with Rheumatoid Arthritis: Results of a Cross-sectional Study.J Rheumatol2019; 46(1):19-26. doi: 10.3899/jrheum.171287.[3]Lin MC, Lu MC, Livneh H, Lai NS, Guo HR, Tsai TY. Factors associated with sexual dysfunction in Taiwanese females with rheumatoid arthritis.BMC Womens Health2017; 17(1):12. doi: 10.1186/s12905-017-0363-5.[4]Frikha F, Masmoudi J, Saidi N, Bahloul Z. Sexual dysfunction in married women with Systemic Sclerosis.Pan Afr Med J2014; 17:82. doi: 10.11604/pamj.2014.17.82.3833.Disclosure of Interests:None declared

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Risk of digital ulcers occurrence in systemic sclerosis: a cross-sectional study

Advances in Rheumatology ◽

10.1186/s42358-019-0057-9 ◽

2019 ◽

Vol 59 (1) ◽

Cited By ~ 3

Author(s):

Alex Magno Coelho Horimoto ◽

Albert Schiaveto de Souza ◽

Silvia Helena Rodrigues ◽

Cristiane Kayser

Keyword(s):

Systemic Sclerosis ◽

Cross Sectional Study ◽

Digital Ulcers ◽

Sectional Study ◽

Cross Sectional

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Gender-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study from the National Registry of the Italian Society Of Rheumatology

The Journal of Rheumatology ◽

10.3899/jrheum.210794 ◽

2021 ◽

pp. jrheum.210794

Author(s):

Rossella De Angelis ◽

Dilia Giuggioli ◽

Gianluigi Bajocchi ◽

Lorenzo Dagna ◽

Giovanni Zanframundo ◽

...

Keyword(s):

Systemic Sclerosis ◽

Demographic Data ◽

Left Ventricular ◽

Sicca Syndrome ◽

Left Ventricular Ejection ◽

Female Group ◽

Special Focus ◽

Digital Ulcers ◽

Cross Sectional ◽

Ventricular Ejection Fraction

Objective There is still a great deal to learn about the influence of gender in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying gender differences in disease expressions, with a special focus on demographic, clinical and serological characteristics. Methods A multicenter SSc cohort of 2,281 patients, 247 men, was recruited in the Italian SPRING (Systemic Sclerosis PRogression INvestiGation) registry. Demographic data, disease manifestations, serological profile and internal organ involvement were compared. Results The overall female/male ratio was 8.2/1. Female/male ratios for limited SSc, diffuse SSc and sine SSc subsets were 8.7/1, 4.9/1, and 10.7/1 respectively. A shorter Raynaud's onset to SSc diagnosis, an increased prevalence of diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, while a significant higher percentage of sicca syndrome, serum ANA, anti-ENA, anti-La/SSB, and anti-CENP-1 was detected in the female group. Males exhibited lower left ventricular ejection fraction, higher prevalence of conduction blocks, arrhythmias, ground glass and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. Conclusion Our study further supports the presence of several gender-related differences in SSc patients. These differences were pronounced as regards the severity of cutaneous, peripheral vascular and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterize the female gender.

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Multiple External Root Resorption of Teeth as a New Manifestation of Systemic Sclerosis—A Cross-Sectional Study in Japan

Journal of Clinical Medicine ◽

10.3390/jcm8101628 ◽

2019 ◽

Vol 8 (10) ◽

pp. 1628 ◽

Cited By ~ 1

Author(s):

Memida ◽

Matsuda ◽

Kajiya ◽

Mizuno ◽

Ouhara ◽

...

Keyword(s):

Systemic Sclerosis ◽

Root Resorption ◽

Temporomandibular Disorder ◽

Mouth Opening ◽

Digital Ulcers ◽

Oral Manifestations ◽

X Rays ◽

Cross Sectional ◽

Facial Region ◽

External Root Resorption

Background: Multiple external root resorption (MERR) has been reported in systemic sclerosis (SSc) patients in Japan and Spain. To establish whether MERR is a new manifestation, we investigated the prevalence of MERR and systemic and oral manifestations to be associated with MERR in patients with SSc. Methods: Root resorption was detected by dental X-rays, panoramagraphy or cone beam computed tomography (CBCT). The prevalence of systemic and oral manifestations was examined by rheumatologists and dentists, respectively. Autoantibodies were investigated using laboratory tests. Results: MERR was detected in four out of the 41 patients (9.8%) who participated in the present study. The prevalence of digital ulcers was significantly higher in patients with MERR (MERR vs non-MERR, 75% vs 16.2%, p < 0.05), whereas that of other systemic manifestations was not. The prevalence of face skin sclerosis (100% vs 10.8%, p < 0.01), calcinosis at the facial region (75% vs 0%, p < 0.01), limited mouth opening (75% vs 18.9% p < 0.05), temporomandibular disorder symptoms (50% vs 2.7%, p < 0.05), and tongue rigidity (75% vs 2.7%, p < 0.05) was significantly higher in patients with MERR. Conclusion: SSc patients with MERR had highly homogenous maxillofacial manifestations. Further clinical and basic studies are needed to elucidate the mechanisms underlying MERR in SSc patients.

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